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Summary: Deficiency of CARD9 (caspase recruitment domain‐containing protein 9) has been reported in individuals with recurrent and invasive fungal infections. We report on a patient who first had Trichosporon asahii affecting the skin then Candida albicans infections involving the digestive tract and knee joint, along with elevated serum IgE. After stimulation with C. albicans, peripheral blood mononuclear cells of this patient produced less tumour necrosis factor‐α, interferon‐γ and interleukin‐17 than those of healthy controls. Furthermore, the serum IgE levels of this patient were positively correlated with the severity of fungal infection during the course of treatment. Sanger sequencing identified one homozygous frameshift mutation (p.D274fsX60) in CARD9. We further performed a review including 48 cases with CARD9 deficiency. According to the data published previously, CARD9‐deficient patients demonstrated obviously elevated IgE in serum (median 1300 IU mL−1), which could distinguish them from otherwise healthy people with fungal infections (area under the curve 0·94, P < 0·001). Patients carrying the mutations Q289X and Q295X had a higher mortality rate (24% vs. 0%, P < 0·05). Patients with the mutations R18W, R35Q, R70W, G72S or Y91H in the CARD domain, and the nonsense mutation Q295X in the coiled‐coil domain, seemed to be more prone to Candida infections (90% vs. 20%, P < 0·005) and central nervous system infections (60% vs. 12%, P < 0·005). What's already known about this topic?: CARD9 deficiency is a rare autosomal recessive genetic defect in T helper 17 immunity, resulting in susceptibility to recurrent and invasive fungal infections.Patients with genetic defects in T helper 17 immunity are prone to hyper‐IgE syndrome. What does this study add? We report a patient with CARD9 p.D274fsX60 mutation manifested as multiple organs infections by Trichosporon asahii and Candida albicans.This study enriches the phenotypic spectrum of CARD9 deficiency.IgE level seems to be a possible clue for diagnosis or therapeutic effect of fungal infection in CARD9‐deficient patients. [ABSTRACT FROM AUTHOR]